Congenital Bilateral Mid Ureteral Stenosis: A Rare Finding

摘要

Bilateral congenital mid ureteric stenosis is a very rare entity causing hydroureteronephrosis. The pathophysiology and aetiology of this condition is uncertain. Congenital functional obstruction of the mid ureter has only 17 reported cases, that too of unilateral pathology. We report a case of an 11-year-old male child who was presented with left sided lump in abdomen since one year. On bilateral retrograde ureteropyelography (RGP), bilateral mid ureteric stenosis was documented at the level of pelvic brim. Tc99m Diethylene Triamine Pentacaetic Acid (DTPA) scan showed non functioning left kidney and decreased function and retention on right side. We did a left nephroureterectomy and uretero-ureterostomy without tapering over a Double-J stent (DJ), with excision of stenosed segment on right side. Pathological review of the ureteric segments of both sides revealed proximal dilatation with non-specific thickening of the muscular wall of the stenosed segments of ureter. The differential diagnosis must additionally include ureteral valves and fibro epithelial polyps. Important role of intravenous pyelography in such cases must be emphasized to localize the pathology site. In addition, retrograde ureteropyelography is essential in management of this anomaly to delineate the stenosed segment of the ureter preoperatively, so that the surgeon can be well prepared with final plan of management.